PEDBASE.org - The Pediatric Database - Detailed information of BENIGN FAMILIAL HEMATURIA

BENIGN FAMILIAL HEMATURIA

DEFINITION:

A familial nephritis characterized by benign hematuria.

EPIDEMIOLOGY:

incidence: ?
age of onset:
- ?
risk factors:
- familial - autosomal dominant
  - chrom.#: ?
  - gene: ?

PATHOGENESIS:

1. Background

disorder results in a secondary glomerulopathy
Familial Nephritis and Benign Familial Hematuria may represent a spectrum of abnormality or abnormalities in the formation of the glomerular basement membrane (BM)

CLINICAL FEATURES:

1. Renal Manifestations

1. Hematuria

persistent or recurrent microscopic hematuria
also persistent microscopic with intermittent gross

Note: Diagnosis is one of exclusion and may need to observe patients over a prolonged period because the hematuria is usually intermittent and evolves over many decades.

INVESTIGATIONS:

1. Urinalysis

hematuria, no proteinuria
microscope - glomerular pattern - damaged RBC's & RBC casts

2. Serum

normal complement

3. Renal Biopsy

light microscopy - normal
electron microscopy - normal or thin glomerular capillary basement membrane
immunofluorescence microscopy - normal

MANAGEMENT:

1. Supportive

follow over a prolonged period with regular urinalyses

2. Prognosis

excellent
no underlying renal disease or complications

Pediatric Database - BENIGN FAMILIAL HEMATURIA

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